[Management of congenital thrombotic thrombocytopenic purpura in the era of recombinant ADAMTS13 protein: Recommendations from the Reference Center for Thrombotic Microangiopathies (CNR-MAT)].

Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by a severe deficiency of ADAMTS13, the specific protease that cleaves von Willebrand factor. The congenital form of TTP (cTTP) results from pathogenic variants of the ADAMTS13 gene. cTTP has two peaks of incidence: one in childhood and the other in adulthood, mainly in an obstetric context.

Baseline Characteristics of Adult Patients Treated and Never Treated with Teduglutide in a Multinational Short Bowel Syndrome and Intestinal Failure Registry.

The Short Bowel Syndrome (SBS) Registry (NCT01990040) is a multinational real-world study evaluating the long-term safety of teduglutide in patients with SBS and intestinal failure (SBS-IF) in routine clinical practice. This paper describes the study methodology and baseline characteristics of adult patients who have (ever-treated) or have never (never-treated) received teduglutide. A total of 1411 adult patients (679 ever-treated; 732 never-treated) were enrolled at 124 sites across 17 countries.

[Nutritionnal complications and patients follow-up after bariatric surgery].

NUTRITIONNAL COMPLICATIONS AND PATIENTS FOLLOW-UP AFTER BARIATRIC SURGERYBariatric surgery is the most consistently effective method for sustained weight reduction and can result in a substantial improvement in overall survival in patients with severe obesity. Complex mechanisms underlying metabolic benefits could also drive preventable, but potentially life-threatening, long-term nutritional complications. Consequently, physicians should be familiar with the lifelong monitoring of patients after bariatric surgery and the potential long-term complications in this paradoxical situation where the long-awaited weight loss can lead to severe nutritional complications.

[Short bowel syndrome: From intestinal insufficiency to intestinal adaptation].

The short bowel syndrome results from an extensive intestinal resection. When intestinal function is below the minimum necessary for the absorption of macronutrients, water and electrolytes, short small bowel syndrome is responsible for chronic intestinal failure. The management is then parenteral nutrition.

A Cognitive Aid Improves Adherence to Guidelines for Critical Endotracheal Intubation in the Resuscitation Room: A Randomized Controlled Trial With Manikin-Based In Situ Simulation.

Introduction: Emergency endotracheal intubation (ETI) is a high-risk procedure. Some of its adverse events are life-threatening, and guidelines emphasize the need to anticipate complications by thorough preparation. The emergency department (ED) can be an unpredictable environment, and we tested the hypothesis that a cognitive aid would help the emergency practitioners better follow guidelines.

Photochromic dye-sensitized solar cells with light-driven adjustable optical transmission and power conversion efficiency.

Semi-transparent photovoltaics only allows for the fabrication of solar cells with an optical transmission that is fixed during their manufacturing resulting in a trade-off between transparency and efficiency. For the integration of semi-transparent devices in building, ideally solar cells should generate electricity while offering the comfort for users to self-adjust their light transmission with the intensity of the daylight. Here we report a photochromic dye-sensitized solar cell (DSSC) based on donor-π-conjugated bridge-acceptor structures where the π-conjugated bridge is substituted for a diphenyl-naphthopyran photochromic unit.

Innate Molecular and Cellular Signature in the Skin Preceding Long-Lasting T Cell Responses after Electroporated DNA Vaccination.

DNA vaccines delivered with electroporation (EP) have shown promising results in preclinical models and are evaluated in clinical trials. In this study, we aim to characterize early mechanisms occurring in the skin after intradermal injection and EP of the auxoGTUmultiSIV DNA vaccine in nonhuman primates. First, we show that EP acts as an adjuvant by enhancing local inflammation, notably via granulocytes, monocytes/macrophages, and CD1a-expressing cell recruitment.

[French adaptation of a new score for global assessment of psoriasis severity: The Simplified Psoriasis Index (SPI)].

Objective: Although several scores exist to assess psoriasis severity, most have marked limitations that rule out their use in routine clinical practice. A new score, the Simplified Psoriasis Index (SPI), has recently been developed and validated in adults in Britain for such use. It has separate components for current severity (SPI-s), psychosocial impact (SPI-p) and past history and interventions (SPI-p), and it is suitable for either professional assessment or patient self-assessment.

[Severe immediate hypersensitivity reaction with generalized contact urticaria after cutaneous application of topical permethrin].

Background: Permethrin is a synthetic pyrethroid used as a chemical insecticide that obtained an MA in the management of human scabies in 2014. We report a case of severe immediate hypersensitivity (IH) reaction with generalized contact urticaria secondary to the cutaneous application of 5% permethrin cream (Topiscab®).

Observation: A 44-year-old woman with no personal history of atopy was treated with oral ivermectin, Topiscab® and levocetirizine for suspected scabies.

Alternative Splicing of Controls Regulatory T Cell Effector Functions and Is Associated With Human Atherosclerotic Plaque Stability.

Rationale: Regulatory T (Treg) cells suppress immune responses and have been shown to attenuate atherosclerosis. The Treg cell lineage-specification factor FOXP3 (forkhead box P3) is essential for Treg cells' ability to uphold immunologic tolerance. In humans, FOXP3 exists in several different isoforms, however, their specific role is poorly understood.

[Employment of People with Mental Disorders in Terms of the Policies Developed by the European and International Institutions].

Objectives The aim of this article is to present an overview of the reflections led by various European and international organizations on the employment of people with mental disorders.Methods This study is based on data from websites of international organizations and interviews taken place with a disability specialist at the International Labour Organisation (ILO) and members of the European Commission.Results Unlike the French law of 11 February 2005 which refers expressly to psychic disability, this notion is not explicitly dedicated by various European and international legal rules.

[Implementation Evaluation of the Pare-Chocs Program in High School].

Despite the important increase in the prevalence of depression during adolescence, a low proportion of adolescent presenting elevated depressive symptoms receive school-based mental health services. Moreover, programs implemented in school settings often suffer of a less rigorous implementation, thus limiting their potential effectiveness. The identification of factors influencing implementation fidelity is essential to improve the quality of services.

[Childhood Maltreatment and Mental Health Problems in Adulthood: A National Study of Women in Quebec].

Objective The study aims to estimate the specific contribution of various forms of child maltreatment on the mental health among a Quebeckers' women representative sample. Method A telephone survey was conducted with a representative sample of 1001 women from the province of Quebec (Canada). Different demographic variables, all four child maltreatment forms (sexual, physical, psychological and neglect) and intimate partner violence experienced in the last 12 months were evaluated to explore their links with depression and post-traumatic stress symptoms of the participants at the time of the investigation.

Guanine glycation repair by DJ-1/Park7 and its bacterial homologs.

DNA damage induced by reactive carbonyls (mainly methylglyoxal and glyoxal), called DNA glycation, is quantitatively as important as oxidative damage. DNA glycation is associated with increased mutation frequency, DNA strand breaks, and cytotoxicity. However, in contrast to guanine oxidation repair, how glycated DNA is repaired remains undetermined.

[Mild hemophilia A fortuitously discovered during Henoch-Schönlein purpura].

Henoch-Schönlein purpura is a common form of immunological vasculitis in children. Hemophilia A is a genetic disorder, inherited in a X-linked recessive pattern, and characterized by spontaneous hemorrhage or prolonged bleeding due to factor VIII deficiency. The clinical signs depend on the severity of factor VIII deficiency.

[DEFI-ALPHA cohort and POLYGEN DEFI-ALPHA clinical research hospital programme. A study about clinical, biological and genetics factors associated with the occurrence and the evolution of hepatic complications in children with alpha-1 antitrypsin deficiency].

Introduction: The alpha-1 antitrypsin (α1-AT) deficiency, most frequently caused by homozygosity for the Z variant (SERPINA1: c.1096 G>A; Glu342Lys), can give rise to two clinical patterns: (i) respiratory impairment with emphysema (mainly in adulthood) because of a pulmonary quantitative defect in anti-elastase activity; (ii) hepatic impairment (mainly in childhood) due to the misfolding of the PiZ protein which accumulates in hepatocytes thus providing cytotoxicity.

Current Knowledge: To date, the clinical and genetic factors responsible for the development of major hepatic injuries (fibrosis and portal hypertension) during childhood in PiZ patients are not known.

Skeletal muscle alterations and exercise performance decrease in erythropoietin-deficient mice: a comparative study.

Background: Erythropoietin (EPO) is known to improve exercise performance by increasing oxygen blood transport and thus inducing a higher maximum oxygen uptake (VO2max). Furthermore, treatment with (or overexpression of) EPO induces protective effects in several tissues, including the myocardium. However, it is not known whether EPO exerts this protective effect when present at physiological levels.

Transcriptome analysis of muscle in horses suffering from recurrent exertional rhabdomyolysis revealed energetic pathway alterations and disruption in the cytosolic calcium regulation.

Recurrent exertional rhabdomyolysis (RER) is frequently observed in race horses like trotters. Some predisposing genetic factors have been described in epidemiological studies. However, the exact aetiology is still unknown.